PERIUMBILICAL PSEUDOXANTHOMA ELASTICUM ASSOCIATED WITH CHRONIC-RENAL-FAILURE AND ANGIOID STREAKS - APPARENT REGRESSION WITH HEMODIALYSIS

Pseudoxanthoma elasticum (PXE) is a heritable connective tissue diseas e involving progressive fragmentation and dystrophic calcification of elastic fibers. Periumbilical disease as the exclusive site of cutaneo us involvement is most commonly seen in the rare entity termed periumb ilical perforating pseudoxanthoma elasticum (PPPXE). Patients with thi s disorder are generally obese, middle aged, multiparous black women w ith hypertension. The cutaneous lesions are well-demarcated, hyperpigm ented, periumbilical plaques with keratotic papules on the periphery. Extracutaneous manifestations have rarely been described, We describe a patient with periumbilical PXE associated with chronic renal failure and bilateral angioid streaks. Histopathologic examination demonstrat ed typical calcification of elastic fibers with additional amorphous c alcium deposits in the superficial dermis. Transepidermal elimination was not present. Normalization of the serum calcium-phosphate product resulted in regression of the lesions-both clinically and histopatholo gically. The relation between PPPXE and hereditary PXE is discussed. T he role of chronic renal failure in precipitating PPPXE is considered.